Clinical and pathological investigation of oligomeganephronia,Pediatric Nephrology

当前位置： X-MOL 学术 › Pediatr. Nephrol. › 论文详情

Our official English website, www.x-mol.net, welcomes your feedback! (Note: you will need to create a separate account there.)

Clinical and pathological investigation of oligomeganephronia
Pediatric Nephrology ( IF 2.6 ) Pub Date : 2022-07-21 , DOI: 10.1007/s00467-022-05687-y
Hideaki Kitakado ₁ , Tomoko Horinouchi ₁ , Chika Masuda ₁ , Atsushi Kondo ₁ , Sadayuki Nagai ₁ , Yuya Aoto ₁ , Nana Sakakibara ₁ , Takeshi Ninchoji _{1,

2} , Norishige Yoshikawa ₃ , Kandai Nozu ₁

Affiliation

Background

Oligomeganephronia (OMN) is a rare congenital anomaly involving the kidney and urinary tract, characterized by decreased number and compensatory hypertrophy of the nephron. It is caused by abnormal kidney development during the embryonic period, especially in patients with low birth weight; however, the actual etiology and clinical features remain unknown. We aim to reveal the clinical and pathological characteristics, treatment, and outcome.

Methods

Ten patients diagnosed with OMN between 2013 and 2020 were retrospectively investigated. The data were presented as the median ± interquartile range, and statistical significance was set at p < 0.05.

Results

The age at diagnosis was 14.1 years, the male-to-female ratio was 6:4, and only four cases were born with low birth weight. The estimated glomerular filtration rate (eGFR) was 62.2 mL/min/1.73 m². The glomerulus diameter of OMN patients was significantly larger (217 vs. 154 µm, p < 0.001) in OMN patients, and the number of glomeruli of OMN patients was lower (0.89 vs. 2.05/mm², p < 0.001) than the control group. Eight of the ten cases were identified by urinary screening. Nine patients were treated with renin–angiotensin system (RAS) inhibitors, following which proteinuria successfully decreased or disappeared. Their median eGFR was also stable, 53.3 mL/min/1.73 m².

Conclusions

As few symptoms can lead to OMN discovery, most patients were found during urine screening at school. Kidney dysfunction was observed in all patients at the time of kidney biopsy. Proteinuria has been significantly reduced and the decline rate of eGFR might be improved by RAS inhibitors.

Graphical abstract

"A higher resolution version of the Graphical abstract is available as Supplementary information"

中文翻译：

少肾症的临床和病理学研究

背景

少肾病（OMN）是一种罕见的先天性异常，累及肾脏和泌尿道，其特征是肾单位数量减少和代偿性肥大。它是由于胚胎时期肾脏发育异常引起的，尤其是低出生体重的患者；然而，实际的病因和临床特征仍然未知。我们的目的是揭示临床和病理特征、治疗和结果。

方法

对 2013 年至 2020 年间诊断为 OMN 的 10 例患者进行回顾性调查。数据以中位数±四分位距表示，统计显着性设定为p < 0.05。

结果

诊断年龄14.1岁，男女比例6:4，仅4例低出生体重儿。估计的肾小球滤过率（eGFR）为62.2 mL/min/1.73 m ² 。 OMN 患者的肾小球直径显着大于 OMN 患者（217 vs. 154 µm， p < 0.001），而 OMN 患者的肾小球数量则低于 OMN 患者（0.89 vs. 2.05/mm ² ， p < 0.001）。对照组。十个病例中有八个是通过尿液筛查发现的。九名患者接受了肾素-血管紧张素系统（RAS）抑制剂治疗，随后蛋白尿成功减少或消失。他们的中位 eGFR 也很稳定，为 53.3 mL/min/1.73 m ² 。